Multinodular and vacuolating neuronal tumor

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Multinodular and vacuolating neuronal tumour (MVNT) is a newnewly recognized cytoarchitectural pattern in the recently revised 2016 edition of the WHO classification of CNS tumours.

Radiologically, MVNTs appear as small 'bubbly' indolent subcortical tumours that sometimes present with seizures. These tumours have been most frequently identified in the temporal lobe, although that is likely to be due to that location being more likely to result in seizures than necessarily a predilection for that lobe.1-4 

Epidemiology

The true epidemiology of these tumours is unknown as they have only been recently described and many are asymptomatic and thus likely undiagnosed (or misdiagnosed). Reported cases are mostly in young to middle aged individuals with adult onset epilepsy 1-4. 

Clinical presentation

Many of these lesions are probably asymptomatic and may be found incidentally on imaging for other reasons, and have, over the year, been misinterpreted a variety of other lesions (see differential diagnosis below). In some individuals these tumours are epileptogenic. 

Pathology

Histopathologic hallmark of MVNT consist of of neuroepithelial cells with conspicuous stromal vacuolation arranged in nodules principally within the deep cortical ribbon and superficial subcortical white matter.1-3 Immunostaining is positive for synaptophysin,HuC/HuD neuronal antigens and p62 but were negative for other markers (e.g. IDH1, nestin, NeuN, neurofilament, GFAP and CD34) 1-4. 

Radiographic features

CT

Smaller lesions are difficult to identify, but if seen will appear as non-enhancing low attenuation lesions deep to the cortex in the subcortical white matter. 

MRI

These tumours appear as a cluster of well circumscribed high T2 signal 'bubbles' located predominantly in the subcortical white matter, but can involve overlying cortex 1,3,4. 

  • T1: hypointense to adjacent grey and white matter
  • T1 C+
    • usually no enhancement
    • some faint focal enhancement may be seen 3,4
  • T2: hyperintense to grey and white matter, almost as high as CSF
  • FLAIR: do not suppress

Treatment and prognosis

MVNTs appear to be benign tumours with very indolent biological behaviour which can, if asymptomatic, be followed with imaging alone. In symptomatic patients (epileptic) surgical resection often controls seizures, with no tumour regrowth reported 1-4. 

Differential diagnosis

  • -<p><strong>Multinodular and vacuolating neuronal tumour (MVNT)</strong> is a new recognized cytoarchitectural pattern in the recently revised 2016 edition of the <a href="/articles/who-classification-of-cns-tumours-1">WHO classification of CNS tumours</a>.</p><p>Radiologically, MVNTs appear as small 'bubbly' indolent subcortical tumours that sometimes present with seizures. These tumours have been most frequently identified in the temporal lobe, although that is likely to be due to that location being more likely to result in seizures than necessarily a predilection for that lobe.<sup>1-4</sup> </p><h4>Epidemiology</h4><p>The true epidemiology of these tumours is unknown as they have only been recently described and many are asymptomatic and thus likely undiagnosed (or misdiagnosed). Reported cases are mostly in young to middle aged individuals with adult onset epilepsy <sup>1-4</sup>. </p><h4>Clinical presentation</h4><p>Many of these lesions are probably asymptomatic and may be found incidentally on imaging for other reasons, and have, over the year, been misinterpreted a variety of other lesions (see differential diagnosis below). In some individuals these tumours are epileptogenic. </p><h4>Pathology</h4><p>Histopathologic hallmark of MVNT consist of of neuroepithelial cells with conspicuous stromal vacuolation arranged in nodules principally within the deep cortical ribbon and superficial subcortical white matter.<sup>1-3 </sup>Immunostaining is positive for synaptophysin,HuC/HuD neuronal antigens and p62 but were negative for other markers (e.g. IDH1, nestin, NeuN, neurofilament, GFAP and CD34) <sup>1-4</sup>. </p><h4>Radiographic features</h4><h5>CT</h5><p>Smaller lesions are difficult to identify, but if seen will appear as non-enhancing low attenuation lesions deep to the cortex in the subcortical white matter. </p><h5>MRI</h5><p>These tumours appear as a cluster of well circumscribed high T2 signal 'bubbles' located predominantly in the subcortical white matter, but can involve overlying cortex <sup>1,3,4</sup>. </p><ul>
  • +<p><strong>Multinodular and vacuolating neuronal tumour (MVNT)</strong> is a newly recognized cytoarchitectural pattern in the recently revised 2016 edition of the <a href="/articles/who-classification-of-cns-tumours-1">WHO classification of CNS tumours</a>.</p><p>Radiologically, MVNTs appear as small 'bubbly' indolent subcortical tumours that sometimes present with seizures. These tumours have been most frequently identified in the temporal lobe, although that is likely to be due to that location being more likely to result in seizures than necessarily a predilection for that lobe.<sup>1-4</sup> </p><h4>Epidemiology</h4><p>The true epidemiology of these tumours is unknown as they have only been recently described and many are asymptomatic and thus likely undiagnosed (or misdiagnosed). Reported cases are mostly in young to middle aged individuals with adult onset epilepsy <sup>1-4</sup>. </p><h4>Clinical presentation</h4><p>Many of these lesions are probably asymptomatic and may be found incidentally on imaging for other reasons, and have, over the year, been misinterpreted a variety of other lesions (see differential diagnosis below). In some individuals these tumours are epileptogenic. </p><h4>Pathology</h4><p>Histopathologic hallmark of MVNT consist of of neuroepithelial cells with conspicuous stromal vacuolation arranged in nodules principally within the deep cortical ribbon and superficial subcortical white matter.<sup>1-3 </sup>Immunostaining is positive for synaptophysin,HuC/HuD neuronal antigens and p62 but were negative for other markers (e.g. IDH1, nestin, NeuN, neurofilament, GFAP and CD34) <sup>1-4</sup>. </p><h4>Radiographic features</h4><h5>CT</h5><p>Smaller lesions are difficult to identify, but if seen will appear as non-enhancing low attenuation lesions deep to the cortex in the subcortical white matter. </p><h5>MRI</h5><p>These tumours appear as a cluster of well circumscribed high T2 signal 'bubbles' located predominantly in the subcortical white matter, but can involve overlying cortex <sup>1,3,4</sup>. </p><ul>
  • -<li>often has <a href="/articles/bright-rim-sign-in-dnets-1">bright FLAIR rim</a>
  • +<li>often has <a href="/articles/bright-rim-sign-in-dysembryoplastic-neuroepithelial-tumours">bright FLAIR rim</a>

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